Incontinentia pigmenti mimicking a herpes simplex virus infection in the newborn

Introduction Incontinentia pigmenti is a rare, X-linked dominant multisystem genodermatosis that presents at or soon after birth with characteristic cutaneous signs. The main features occur in the skin where a blistering rash occurs in the newborn period, followed by the blisters becoming raised-like warts. After the skin, the central nervous system is the next most affected system. Case report We report a female infant who was born with vesicular eruptions who was initially thought to have congenital herpes simplex virus infection. Conclusion This case report emphasises that incontinentia pigmenti should be included in the differential diagnosis of cutaneous blistering lesions and central nervous system involvement in neonates. This case was presented in part at the 22nd International Symposium on Neonatal Intensive Care, Milan, Italy, October, 2006.